Water-Clear Cell Parathyroid Adenoma Causing Primary Hyperparathyroidism in a Patient with Neurofibromatosis Type 1: Report of a Case

Water-clear cell parathyroid adenoma is an exceedingly rare tumor, composed exclusively of tumor cells with abundant foamy cytoplasm. A combination of hyperparathyroidism and neurofibromatosis type 1 (NF1) is also a rare phenomenon. We report an 18-year-old woman with primary hyperparathyroidism caused by water-clear cell parathyroid adenoma in association with NF1. She had renal stones, hypercalcemia, and an elevated plasma level of intact parathyroid hormone. Physical examination revealed a palpable tumor in the right neck, and café-au-lait spots distributed over her entire body. An ultrasound examination showed an isoechoic mass in the right thyroid lobe. Thallium–technetium subtraction scintigraphy showed high thallium accumulation in the right thyroid lobe area. A surgical exploration revealed the palpable mass to be a parathyroid tumor. The pathological features were consistent with water-clear parathyroid adenoma. This is the first reported case of water-clear cell parathyroid adenoma associated with NF1.     

Hyperfunctional parathyroid carcinoma

The parathyroid cancer is mentioned in literature with an incidence of 0.5-5% in the etiology of the primary hyperparathyroidism. The authors present the case of a 45 year old female with diagnosis of "primary hyperparathyroidism" based on clinical, ultrasonographic and biochemical investigations. A right superior adenoma of 3 x 2 x 1 cm (150 mg) has been found and extirpated. The histological examination showed an adenoma with predominant "chief" cells. Three years after surgery the patient had a recurrence of the clinical and hypercalcemie syndrome (with more severe damages). The surgical reexploration showed the right thyroid lobe with a nodular aspect and in its inferior pole a enlarged parathyroid gland of one cm size was found. An en-block exeresis including the right thyroid lobe with isthmus as well as the mentioned lesion and the half of left superior parathyroid, the left inferior one together with retrosternal fat tissue have been performed. Frozen sections completed by paraffin examination established the diagnosis of hyperfunctioning parathyroid carcinoma. We have emphasize the issues that could suggest the initially preoperative true diagnosis: the large size of excised adenoma and the relatively quick recurrence of the phenomena of parathyroid hyperfunction. The surgical principles and strategies in the treatment of parathyroid cancer are also discussed.     

Primary hyperparathyroidism with thyroid hemiagenesis

Thyroid hemiagenesis is a very rare anomaly. We herein report a case with right thyroid lobe agenesis, which was incidentally found during the assessment of primary hyperparathyroidism. A 42-year-old male presenting with urinary lithiasis was suspected of having primary hyperparathyroidism, and had elevated levels of both serum calcium and intact parathyroid hormone. Both computed tomography and ultrasonography demonstrated the absence of right thyroid lobe and a mass of 1 cm in diameter at the left lower pole of the thyroid. The patient underwent lower left parathyroidectomy, which confirmed the right thyroid hemiagenesis, as well as the absence of both upper and lower right parathyroid glands. The resected left lower parathyroid gland was pathologically diagnosed as adenoma. The postoperative course was favourable and he was discharged on the 2nd day after surgery, without complications.     

Intracystic hemorrhage in a mediastinal cystic adenoma causing parathyrotoxic crisis

BACKGROUND: We report a case of intracystic hemorrhage in a mediastinal cystic parathyroid adenoma causing parathyrotoxic crisis. METHODS AND RESULTS: A 30-year-old man presented with a large neck mass, dyspnea, and abdominal pain. The patient's serum calcium and parathormone levels were elevated. Radiography showed a right tracheal deviation, ultrasonography identified a thyroid nodular goiter extending to the mediastinum with a large (4.0 cm x 5.6 cm) cystic mass adjacent to the lower left thyroid pole. After IV fluid, pamidronate, and furosemide were administered, the patient underwent total thyroidectomy, and excision of the cyst and a small mass (2 cm x 2 cm) adjacent to the upper right thyroid lobe. Histopathologic examination revealed a double parathyroid adenoma and identified the mediastinal lesion as a cystic adenoma with intracystic hemorrhage. CONCLUSIONS: Intracystic hemorrhage in a functional mediastinal cystic parathyroid adenoma is an extremely rare cause of parathyrotoxic crisis. Aggressive medical treatment should be immediately instituted, and surgery should be performed as soon as hypercalcemia is controlled.     

Parathyroid cytology: avoiding diagnostic pitfalls

BACKGROUND: Interpretation of parathyroid fine-needle aspirates (FNA) remains problematic not only because this type of specimen is rare but also because the pertinent literature is very limited. We systematically reviewed parathyroid FNAs in our files and sought to delineate additional diagnostic criteria. DESIGN: Review of all thyroid and parathyroid aspirates from January 1990 to June 1998 disclosed 12 parathyroid lesions. The final diagnoses included four parathyroid adenomas, one intrathyroidal hyperplastic parathyroid, one intrathyroidal parathyroid adenoma, one atypical parathyroid adenoma (all confirmed by histologic screening or immunocytochemistry), and five parathyroid cysts (all confirmed by immunoassay). Papanicolaou and Diff Quik-stained smears of the parathyroid FNAs were reviewed. The cytologic features were compared and contrasted with those of thyroid FNAs to establish criteria for differential diagnoses. RESULTS: The FNAs of the five parathyroid cysts yielded virtually acellular fluid with a characteristic water-clear appearance and markedly elevated levels of parathyroid hormone. The remaining seven aspirates consisted of moderately cellular smears that showed an admixture of architectural features. Common patterns included cohesive three-dimensional groups, disorganized sheets, papillary fragments, microfollicles, and a single case showing lymphoidlike smears. Although the cells were generally small and round to oval, all cases demonstrated mild to moderate anisokaryosis. The nuclei were hyperchromatic E with coarsely granular chromatin reminiscent of that of small lymphocytes. Occasional nucleoli were noted. Although the cytoplasm was usually pale blue and finely granular with ill-defined borders, two cases showed well-delineated cytoplasmic membranes. Less common findings included cytoplasmic granulation, vacuolization, and rare oxyphilic cells. Naked nuclei were noted in the background of all of the aspirates to varying degrees. Other background findings included the presence of colloidlike material, macrophages, and lymphocytes. One interesting finding that to date has not been reported is the presence of nuclear overlapping (100%) and nuclear molding (71%), which is an uncommon finding in thyroid aspirates. CONCLUSIONS: FNAs of the parathyroid can be easily confused with that of the thyroid, not only because of the clinical similarity between these two types of lesions but also because of the overlap in cytomorphologic features of the aspirated cells. Although no one single cytomorphologic feature is diagnostic, a combination of cytologic parameters noted earlier should raise the possibility of a parathyroid lesion. Aspirates of parathyroid cysts show acellular water-clear fluid with elevated parathyroid hormone measurements.     

GIANT DOUBLE PARATHYROID ADENOMA PRESENTING AS A HYPERCALCAEMIC CRISIS

The largest documented case of a double parathyroid adenoma is reported. The patient presented in hypercalcaemic crisis with a large intrathoracic mass. After removal of a massive cystic parathyroid adenoma from the right superior mediastinum. a second very large parathyroid adenoma was found on the contralateral side adjacent to the left thyroid lobe. This case illustrates the importance of the cervical approach, as well as routine bilateral neck exploration. for all cases of primary hyperparathyroidism.     

Case Report on Giant Parathyroid Adenoma with Review of Literature

Symptomatic primary hyperparathyroidism is still predominant form in developing countries. Sumit Shukla et al. (2008) Most of these lesions are usually less than 2 cm in diameter with serum calcium levels between 11 to 13 mg/dl. Our case had 3.9?×?2?×?1.7 cm non palpable right inferior parathyroid adenoma extending into superior mediastinum, serum calcium 17 mg/dl and vomiting as presenting symptom a case report.     

A case of secondary hyperparathyroidism with an ectopic intrathyroid gland successfully diagnosed and controlled by percutaneous ethanol injection therapy (PEIT)

Secondary hyperparathyroidism (II HPT) is a major complication in chronic dialysis patients, and percutaneous ethanol injection therapy (PEIT) has become a useful alternative treatment for II HPT. However, the existence of ectopic parathyroid glands is a major problem when conducting PEIT. Ectopic parathyroid gland accepts 10-35% of II HPT, and the missing glands cannot be detected consistently by any imaging techniques, including scintigraphy. Intrathyroid parathyroid gland is as rare as about 1% and recurrence of missing glands after parathyroidectomy (PTx) has been reported in some cases. We report here a 52-year-old female in whom an ectopic parathyroid gland was defected successfully and intact-PTH controlled by tentative PEIT. At the first examination, a left parathyroid adenoma and a right thyroid goiter were pointed out by ultrasonography, CT and scintigraphy. PEIT was applied twice to the left parathyroid adenoma, but intact-PTH was not decreased. Ultrasonography, CT, 201Tl-99mTc subtraction scintigraphy and fine needle aspiration biopsy (FNAB) were performed again to search for the existence of ectopic glands. The results suggested that the right intrathyroid tumor was an ectopic parathyroid gland. Consequently, tentative PEIT was applied to the right intrathyroid tumor, and successful control of intact-PTH and serum Ca was eventually achieved. To our knowledge, this is the first reported case of secondary hyperparathyroidism with an ectopic intrathyroid gland that was successfully controlled by PEIT. In this case, it was suggested that tentative PEIT of intrathyroid tumor was a useful method for detecting an ectopic parathyroid gland.     

A parathyroid cyst and an adenoma associated with primary hyperparathyroidism: report of a case

A case of a parathyroid cyst associated with an adenoma in a different gland is reported. A 55-year-old female was explored with preoperative diagnosis of primary hyperparathyroidism after endoscopic removal of a right ureteral stone. The operation revealed a cyst, 20 x 10 mm in size at the left lower gland, and a solid tumor, 32 x 12 x 7 mm in size and 1,300 mg in weight in the left upper gland. Histological examination disclosed a parathyroid cyst and an oxyphilic and chief cell adenoma, respectively. Postoperative course was uneventful including normalized serum Ca level. To our knowledge, 59 cases of parathyroid cysts have been reported in the Japanese literature, of which 31 were in the hyperparathyroid status. The most common causes were cystic degeneration of the adenomas. A parathyroid cyst with a concomitant adenoma in a different gland like our case is very rare. This is the first reported case in Japan and the sixth reported case in the world literature.     

Needle aspiration of nonfunctioning parathyroid cysts

Until recently, nonfunctioning parathyroid cysts were usually identified at operation for a presumed thyroid mass. Thyroid needle biopsy now allows their preoperative diagnosis and potential definitive treatment. This study reviews four patients with nonfunctioning parathyroid cysts treated during a two-year period. Three women and one man range in age from 28 to 70 years. Each presented with an asymptomatic thyroid mass ranging from 3 to 5 cm in length. None had symptoms of primary hyperparathyroidism. Serum calciums were from 9.2 to 10.7 mg/dl and serum phosphoruses were 3.2 to 4.4 mg/dl. Needle aspiration revealed 5 to 85 cc of water-clear fluid. C-terminal parathyroid hormone in three patients was 12,600, 6,500 and 61,200 pg/ml and N-terminal PTH was 1,700 pg/ml in one. All four had normal serum calcium and phosphorus on follow-up ranging from six months to two years. Two patients had resolution of their cysts with a single aspiration. One patient had recurrence but has no evidence of recurrence six months after injection with tetracycline. Another patient had a recurrence but remains well one year following reaspiration. Nonfunctioning parathyroid cysts present as a thyroid mass. Needle aspiration of water-clear fluid high in parathormone is diagnostic and, in most patients, is the therapeutic modality of choice.     

Surgical strategy for primary hyperparathyreoidism with thyroid hemiagenesis

Background

Thyroid hemiagenesis is a rare congenital anomaly, and still more rarely associated with primary hyperparathyroidism (pHPT). Due to the embryologic pathways of the thyroid and parathyroid glands, it remains unclear whether or not thyroid hemiagenesis may be linked to ipsilateral parathyroid agenesis, and consequently, surgical strategy for thyroid hemiagenesis associated pHPT (THAP) does not only depend on preoperative localization but also on the thyroid anomaly.

Methods

Including the present case report, a total of nine cases with THAP retrieved from the literature were reviewed. Seven of nine cases had thyroid hemiagenesis on the left side, three out of nine showed a parathyroid adenoma on the contralateral side to the thyroid hemiagenesis.

Conclusions

Based on these cases, it can be concluded that the embryologic pathways of the thyroid and parathyroid glands are different, and in cases of THAP, parathyroid exploration should follow standard recommendations for pHPT surgery.     

Occult fifth gland intrathyroid parathyroid adenoma identified by gamma probe

We report the case of a 54-year-old woman undergoing radioguided parathyroidectomy in whom four normal glands were identified on cervical exploration. The gamma probe was instrumental in guiding us to the adenoma in a fifth gland that was not visible and was localized to the parenchyma of the left lobe of the thyroid gland. Many surgeons do not advocate use of the gamma probe in minimally invasive parathyroidectomy and rely solely on focused exploration on the basis of a preoperative sestamibi scan and intraoperative rapid parathyroid hormone assays. This case, however, illustrates the utility of the gamma probe in parathyroid adenoma localization.     

Water clear cell ectopic non-iatrogenic giant parathyroid adenoma in sternohyoid muscle with thyroid nodule and asymptomatic hypercalcemia due to primary hyperparathyroidism: Case report and literature review

BackgroundEctopic parathyroid gland is not uncommon, and is associated with primary hyperparathyroidism. Giant parathyroid adenoma (PA) and concurrent presence of enlarged thyroid nodule increases the probability of ectopic location. The combination of a giant PA that is ectopic (within the strap muscle) in the neck is very rare, especially in cases with no previous surgery. The rare histopathological findings of the current case, water clear cell parathyroid adenoma (WCCPA), could explain the patient's presentation, since it has low endocrine function.Case presentationA 56-year-old Qatari female on routine visit to primary health care physician for hypertension, was incidentally discovered to be hypercalcemic and was referred to the emergency department of our institution. Neck ultrasound showed a thyroid nodule on the left side, but ^99mTc-sestamibi scintigraphy identified a left PA. FNAC of the thyroid nodule showed that it was a colloid nodule. She underwent left hemithyroidectomy and excision of left PA. Intraoperatively, the PA was giant and in the sternohyoid muscle. Intraoperative monitoring of intact PTH (IOiPTH) confirmed successful excision.DiscussionEctopic giant parathyroid adenoma is rare especially with the intramuscular location in sternohyoid muscle in the neck without previous neck surgeries. The presence of thyroid nodule could be a precipitating factor for migration of the PA. Preoperative assessment with the radiological image is crucial for diagnosis but sometimes fail to localized the PA.ConclusionGiant asymptomatic PA with long standing low function before hyperfunctioning should raise the suspicion of WCCPA. If diagnosis is confirmed, metastasis from a clear cell renal cell carcinoma should be ruled out.     

Transoral parathyroid surgery—a new alternative or nonsense?

Purpose

In recent years, several endoscopic techniques have been explored in thyroid and parathyroid surgery, but only few gained acceptance among patients and surgeons. Based on extensive human cadaver and animal studies, we developed a technique for transoral partial parathyroidectomy (TOPP), which was performed for the first time in a patient with primary hyperparathyroidism (pHPT). We now report on results and the acceptance of this new technique 2 years after its implementation.

Methods

A pilot study was initiated to recruit a total of 10 patients with benign sporadic pHPT and a preoperatively localized parathyroid adenoma eligible for initial parathyroidectomy. The study protocol was approved by the ethics committee, and an insurance for unforeseen complications and risks was procured. Data of all patients evaluated and operated were prospectively collected, and follow-up examinations were carried out for 19 months on average, which included clinical examinations; ultrasonography; Ear, Nose, and Throat (ENT) investigations; and blood testing.

Results

Between January 2010 and May 2012, 75 patients with pHPT and a preoperative localized parathyroid adenoma were eligible for TOPP. After detailed information about the transoral procedure, only five (7 %) female patients consent to undergo TOPP. In three patients, a parathyroid adenoma could be removed via the transoral access, In two patients, the procedure had to be converted to the conventional technique. Median time until resection of a parathyroid adenoma was 122 min (range, 45–175). One patient had a transient recurrent laryngeal nerve palsy, while one patient suffered from a transient palsy of the right hypoglossal nerve and a slight but persisting dysgeusia. Three patients developed a hematoma of the mouth floor and swallowing problems. In four patients, the visual analog scale (VAS) pain score was high (>7) within the first 2 postoperative days.

Conclusions

Although TOPP is feasible, it is poorly accepted by patients and its complication rate is high. Thus, TOPP is nonsense with currently available devices.     

甲状旁腺肿瘤合并甲状旁腺功能亢进症诊治经验

目的 总结甲状旁腺肿瘤合并甲状旁腺功能亢进症的诊治体会。方法 回顾性分析我院2007年2月至2012年2月收治的4例甲状旁腺肿瘤合并甲状旁腺功能亢进症的临床特征、诊断过程、治疗情况与治疗效果。结果 以多发性骨折及颈前肿块为首发症状者各2例,入院时3例被误诊（误诊率75%,3/4）,3例并存泌尿系结石及甲状腺疾病;受检者全部存在高血钙、低血磷、高尿钙及甲状旁腺激素升高;术前B超、CT检查与术中及术后病理诊断符合率分别为75%及100%;术中见2例位于甲状腺右叶下极后下方,1例位于甲状腺左叶下极后下方,另1例位于左叶甲状腺腺体内;3例良性者（甲状旁腺腺瘤）行局部切除,1例恶性者（甲状旁腺癌）行左甲状腺全切除;术后早期均出现一过性低钙低钾低镁血症,2周内电解质、尿钙、甲状旁腺激素水平均恢复正常。结论 此病极易误诊,术前必须注重定性及定位诊断,外科手术切除是首选的治疗方法。     

An ectopic parathyroid gland in the left axillary region: case report

This is a review of ectopic localizations of the parathyroid gland in one case. A hard mobile mass (2 x 2.5 cm) was detected on the right side of the neck of a patient who had previously been given a diagnosis of hyperparathyroidism. Computed tomography showed an adenoma (25 x 35 mm) in the paratracheal region on the right side of the neck. Scintigraphy revealed an activity increase on the right side of the neck and in the left axillary region. Blood measurements were as follows: Ca, 12.7 mEq/l; parathyroid hormone (PTH), 235 U/l; and alkaline phosphatase (ALP), 776 U/l. The patient was operated on to remove the lesions located in the cervical and left axillary regions. Histopathological examination revealed that the lesion in the right cervical region was a parathyroid adenoma, whereas the lesion in the left axillary region was normal parathyroid gland tissue. Parathyroid glands may indicate ectopic locations in very different regions. We think the case presented here is the first case with this axillary location. Scintigraphy, alone or combined with other methods, may be used to determine ectopic localizations.     

A case of functioning parathyroid cyst]

We report a rare case of primary hyperparathyroidism with a functioning parathyroid cyst in a 45-year-old male. He was a recurrent stone former, and consulted our hospital for further examinations of hypercalcemia. Plasma levels of intact parathyroid hormone (PTH) were elevated to 130 pg/ml. Ultrasonography, computed tomography and magnetic resonance imaging revealed a parathyroid cyst on the right lobe of the thyroid gland. We performed right superior parathyroidectomy. Histological examination demonstrated a secondary pseudocyst resulting from cystic degeneration of a parathyroid adenoma. Plasma levels of intact PTH normalized after operation. To our knowledge, only 48 cases of functioning parathyroid cyst were reported in the Japanese literature. We discuss the clinical features and histological evidence of functioning parathyroid cyst.     

Primary hyperparathyroidism in children

We present here a series of seven children with primary hyperparathyroidism caused by parathyroid adenoma. Chief cells were the primary element in six patients and water-clear cells in one patient. A brief review of the literature on primary hyperparathyroidism in children is included. Emphasis is placed on the clinical characteristics of this rare disease in children.     

Ectopic atypical parathyroid lipoadenoma: a rare cause of severe primary hyperparathyroidism

Parathyroid lipoadenomas are a rare and benign variant of parathyroid adenomas that are defined morphologically by an abundance of fat cells. They may be functional with secretion of parathyroid hormone or nonfunctional. We present a case of a parathyroid lipoadenoma in a patient presenting with clinical manifestations of severe primary hyperparathyroidism. Unusual features in this case included the ectopic location of the lipoadenoma in the mediastinum, the significant size of 6.6 × 3.4 cm, and weight of 30.1 g. In addition to the large size of this generally benign type of parathyroid neoplasm, histologic findings included focal band-forming fibrosis compatible with the diagnosis of an atypical parathyroid adenoma. In conclusion, this case illustrates a difficult case of an ectopic functioning parathyroid lipoadenoma with chronic sequelae due to severe hyperparathyroidism.     

A case of parathyroid carcinoma

A case of parathyroid carcinoma in a 49-year-old female is described. She is found to have renal stones and high serum calcium levels. On physical examination a fixed walnut-sized mass was detected on her right neck. Laboratory studies disclosed a serum calcium level of 12.7 mg/dl, serum phosphorus level of 2.5 mg/dl, serum Ca++ level of 3.12 mEq/l, and positive Gold-Smith test. Computer-assisted tomography scan and ultrasonogram showed a mass in the posterior region of the right lobe of her thyroid. The tumor was resected and histopathological examination showed parathyroid carcinoma. The patient had no evidence of recurrence 2 years following surgery. Our report is the 70th case among Japanese literature.